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Eponyms highlight the contributions made to medicine over the years, and celebrate individuals for their work involving diseases, pathologies, and anatomical landmarks. We have compiled an in-depth report of eponyms used in skull base neurosurgery, as well as the historical contexts of the personalities behind the names. A literature search identified 36 eponyms of the bones, foramina and ligaments of the skull base named after anatomists and physician-scientists. The 36 eponymous structures pinpointed include Arnold's canal, the foramen of Arnold, Bill's bar, Bertin's bones, Civinini's canal, Civinini's ligament, Civinini's process, sinodural angle of Citelli, Clivus of Blumenbach, Dorello's canal, the Eustachian tube, the eponymous cavernous sinus triangles of Parkinson, Kawase, Mullan, Dolenc, Glasscock and Hakuba, the Fallopian canal, the Glasserian fissure, Gruber's ligament, Haller cells, the spine of Henle, Highmore's antrum, the foramen of Huschke, Hyrtl's fissure, the Ingrassia process, Jacobson's canal, the MacEwen triangle, Meckel's cave, the Onodi air cell, the Pacchionian foramen, Fossa of Rosenmuller, the foramen of Vesalius, the Vidian canal, Trautman's triangle and the annular tendon of Zinn. Knowledge of the relevant eponyms enables succinct descriptions of important skull base structures, provides an understanding of associated clinical implications, and reminds us of the vast history of contributions to neurosurgery made by prominent figures in the field.

Background: Gender-affirming hormone therapy is critical to the management of transgender persons. Cyproterone acetate (CPA) is a synthetic, progesterone-like compound commonly used in high doses a...

Due to the risk of intracranial aneurysm (IA) recurrence and the potential requirement for re-treatment following endovascular treatment (EVT), radiological follow-up of these aneurysms is necessary. There is little evidence to guide the duration and frequency of this follow-up. The aim of this study was to establish the current practice in neurosurgical units in the UK and Ireland.A survey was designed with input from interventional neuroradiologists and neurosurgeons. Neurovascular consultants in each of the 30 neurosurgical units providing a neurovascular service in the UK and Ireland were contacted and asked to respond to questions regarding the follow-up practice for IA treated with EVT in their department.Responses were obtained from 28/30 (94%) of departments. There was evidence of wide variations in the duration and frequency of follow-up, with a minimum follow-up duration for ruptured IA that varied from 18 months in 5/28 (18%) units to 5 years in 11/28 (39%) of units. Young patient age, previous subarachnoid haemorrhage and incomplete IA occlusion were cited as factors that would prompt more intensive surveillance, although larger and broad-necked IA were not followed-up more closely in the majority of departments.There is a wide variation in the radiological follow-up of IA treated with EVT in the UK and Ireland. Further standardisation of this aspect of patient care is likely to be beneficial, but further evidence on the behaviour of IA following EVT is required in order to inform this process.

Gross total resection remains the gold-standard approach for vestibular schwannomas (VS) when surgery is indicated. In select cases, incomplete resection (IR) becomes a desired alternative to preserve the facial nerve function and the patient's quality of life. While a lot of earlier studies described incompletely resected sporadic VSs as dormant, more recent studies reported a higher growth rate following IR, therefore an evaluation of the residual VS growth rates could have important implications for the follow-up treatment protocols and provide relevant information for neurosurgeons, neuro-otologists, neuropathologists, and radiologists. Although prognostic factors predicting preoperative VS growth have been previously investigated, these factors have not been investigated following IR. Our review aims to examine the growth rate of residual sporadic VS following IR and to examine variables associated with the regrowth of residual VS.The review was conducted in accordance with the PRISMA guidelines. Six databases (MEDLINE (Ovid), Embase (Ovid), CINAHL Plus (EBSCO), Cochrane Central Register of Controlled Trials (CENTRAL), WHO International Clinical Trials Registry Platform and UK Clinical Trials Gateway (WHO ICTRP) were searched. Full-text articles analysing growth rates in at least ten patients who had residual VS after IR were assessed. We conducted a meta-analysis using a random-effects model via RevMan.14 studies totalling 849 patients were included in the analysis. The mean planimetric growth rate was 1.57 mm/year (range 0.16-3.81 mm/year). The mean volumetric growth rate was 281.725 mmFrom our analysis, only the residual tumour volume/size was associated with residual VS growth. Therefore, close postoperative surveillance for the first year, followed by an annual MRI scan for at least 5 years, and subsequently extended interval surveillance remains of utmost importance to monitor disease progression and provide timely surgical and adjuvant interventions. Our study shows that future work should be aimed at molecular and histological characteristics of residual VSs to aid prognostic understanding of growth.

Intracranial aneurysms are a common asymptomatic vascular pathology, the rupture of which is a devastating event with a significant risk of morbidity and mortality. Aneurysm detection and risk stratification before rupture events are, therefore, imperative to guide prophylactic measures. Artificial intelligence has shown great promise in the management pathway of aneurysms, through automated detection, the prediction of rupture risk, and outcome prediction after treatment. The complementary use of these programs, in addition to clinical practice, has demonstrated high diagnostic and prognostic accuracy, with the potential to improve patient outcomes. In the present review, we explored the role and limitations of deep learning, a subfield of artificial intelligence, in the aneurysm patient journey. We have also briefly summarized the application of deep learning models in automated detection and prediction in cerebral arteriovenous malformations and Moyamoya disease.

Aneurysmal subarachnoid hemorrhage (aSAH) is a devastating form of stroke frequently affecting young to middle-aged adults, with an unmet need to improve outcome. This special report focusses on the development of intrathecal haptoglobin supplementation as a treatment by reviewing current knowledge and progress, arriving at a Delphi-based global consensus regarding the pathophysiological role of extracellular hemoglobin and research priorities for clinical translation of hemoglobin-scavenging therapeutics. After aneurysmal subarachnoid hemorrhage, erythrocyte lysis generates cell-free hemoglobin in the cerebrospinal fluid, which is a strong determinant of secondary brain injury and long-term clinical outcome. Haptoglobin is the body’s first-line defense against cell-free hemoglobin by binding it irreversibly, preventing translocation of hemoglobin into the brain parenchyma and nitric oxide-sensitive functional compartments of cerebral arteries. In mouse and sheep models, intraventricular administration of haptoglobin reversed hemoglobin-induced clinical, histological, and biochemical features of human aneurysmal subarachnoid hemorrhage. Clinical translation of this strategy imposes unique challenges set by the novel mode of action and the anticipated need for intrathecal drug administration, necessitating early input from stakeholders. Practising clinicians (n=72) and scientific experts (n=28) from 5 continents participated in the Delphi study. Inflammation, microvascular spasm, initial intracranial pressure increase, and disruption of nitric oxide signaling were deemed the most important pathophysiological pathways determining outcome. Cell-free hemoglobin was thought to play an important role mostly in pathways related to iron toxicity, oxidative stress, nitric oxide, and inflammation. While useful, there was consensus that further preclinical work was not a priority, with most believing the field was ready for an early phase trial. The highest research priorities were related to confirming haptoglobin’s anticipated safety, individualized versus standard dosing, timing of treatment, pharmacokinetics, pharmacodynamics, and outcome measure selection. These results highlight the need for early phase trials of intracranial haptoglobin for aneurysmal subarachnoid hemorrhage, and the value of early input from clinical disciplines on a global scale during the early stages of clinical translation.

Background Africa bears >15% of the global burden of neurosurgical disease. Yet to date, Africa still has the lowest neurosurgical workforce density globally, and efforts to fill this gap by 2030 need to be multiplied. Although the past decade has seen an increase in neurosurgery residency programs in the continent, it is unclear how these residency programs are similar or viable. This study aims to highlight the current status of neurosurgical training in Africa as well as the differences within departments, countries or African regions. Methods A literature search using keywords related to ‘neurosurgery’, ‘training’, and ‘Africa’ and relevant names of African countries will be performed on PubMed and Google Scholar. If unavailable online, the authors will contact local neurosurgeons at identified training programmes for their curricula. The residency curricula collected will be analysed against a standardized and validated medical education curriculum viability tool. Results The primary aim will be the description of African neurosurgical curricula. In addition, the authors will perform a comparative analysis of the identified African neurosurgical curricula using a standardized and validated medical education curriculum viability tool. Discussion This study will be the first to evaluate the current landscape of neurosurgery training in Africa and will highlight pertinent themes that may be used to guide further research. The findings will inform health system strengthening efforts by local training programme directors, governments, policymakers and stakeholders.

Background Vestibular schwannomas (VS) are benign intracranial tumors originating from the vestibular division of the eighth cranial nerve. Treatment options include microsurgery, radiotherapy, and surveillance. Endoscopy is becoming more widely used as an adjunct in skull base surgery and may influence outcomes in surgically managed VS. Methods A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies involving endoscope-assisted and fully endoscopic procedures for sporadic VS resection were identified. Facial nerve function, hearing preservation, extent of resection, and complications were analyzed. Results Thirty-one studies were included (27 endoscope-assisted, four fully endoscopic). Subgroup analyses were performed to assess outcomes according to tumor size and surgical approach. Overall, endoscopic facial nerve preservation rates were comparable to microsurgical treatment. A subgroup analysis suggested that functional facial nerve preservation rates may be higher when endoscopic assistance is used for smaller (Koos I-II) tumors using the retrosigmoid or translabyrinthine approach. The gross total resection rate for small tumors was higher in retrosigmoid ES-assisted microsurgery (96.2%) compared to rates in the literature for the standard, open retrosigmoid approach. Hearing outcomes were more variable and were under-reported. Conclusions Current data suggest that ES-assisted resection of sporadic VS is not inferior to microsurgical resection with respect to facial nerve outcomes and extent of resection. However, some ES series report poor hearing outcomes, which are under-reported in the literature. Further prospective studies are required to ascertain if endoscopic assistance can improve outcomes for VS resection, particularly for smaller (Koos I-II) tumors.

Background There is no comprehensive report of neurosurgery postgraduate education in Africa. This narrative review aimed to map out the landscape of neurosurgery training in Africa and highlight similarities and differences in training. Methods The keywords “neurosurgery,” “education,” and “Africa” were searched on PubMed and Google Scholar from inception to January 17, 2021. Next, a complementary hand search was conducted on Google using the keywords “neurosurgery,” “residency,” and the individual African countries in English and official languages. The relevant data were extracted and compiled into a narrative review. Results A total of 76 African training programs that recruit more than 168 trainees each year were identified. Less than half (40.7%, n = 22) of African countries have at least 1 neurosurgery training program. Egypt (n = 15), Algeria (n = 14), and Nigeria (n = 10) have the highest number of training programs, whereas Algeria (0.33), Egypt (0.15), and Libya (0.15) have the highest number of training programs per 1 million inhabitants. The College of Surgeons of East, Central, and Southern Africa has 16 programs in 8 countries, whereas the West African College of Surgeons has 17 accredited programs in 3 countries. The duration of training varies between 4 and 8 years. There is limited information available in the public domain and academic literature about subspecialty fellowships in Africa. Conclusions This review provides prospective applicants and African and global neurosurgery stakeholders with information to advocate for increased investment in African neurosurgery training programs.

Introduction\ud Africa bears more than 15% of the global burden of neurosurgical disease; however, it has the lowest neurosurgical workforce density worldwide. The past decade has seen an increase in neurosurgery residency programs on the continent. It is unclear how these residency programs are similar or viable. This study highlights the current status, interdepartmental and regional differences, with the main objective of offering a template for improving the provision of neurosurgical education on the continent.\ud \ud Method\ud PubMed and Google Scholar were searched using keywords related to “neurosurgery,” “training,” and “Africa” from database inception to 10/13/2021. The residency curricula were analyzed using a standardized and validated medical education curriculum viability tool.\ud \ud Results\ud Curricula from 14 African countries were identified. The curricula differed in resident recruitment, evaluation mode and frequency, curriculum content, and length of training. The length of training varied from four to eight years with a mean of six years. The Eastern African region had the highest number of examinations, with a mean of 8.5. Few curricula had correlates of viability - ensuring that the instructors are competent (64.3%), prioritization of faculty development (64.3%), faculty participation in decision making (64.3%), prioritization of resident support services (50%), creating a conducive environment for quality education (42.9%), and addressing student complaints (28.6%).\ud \ud Conclusion\ud There are significant differences in the African postgraduate neurosurgical education curriculum warranting standardization. This study has identified areas of improvement for neurosurgical education in Africa.

Surveillance imaging is a valid management option for selected vestibular schwannomas (VS). An ideal protocol for radiologic monitoring would highlight growth-related risk factors and tailor management accordingly. This study aims to identify variables associated with the growth of sporadic VS to enhance surveillance imaging, enable early intervention, and optimize outcomes.The review was conducted according to the PRISMA guidelines. A systematic review of 5 databases (PubMed, Ovid, Cochrane Library, Web of Science, and Google Scholar) was performed to identify negative and positive growth predictors of sporadic vestibular schwannomas. The search was limited to studies reported between January 2015 and January 2020. We conducted an individual patient data meta-analysis using a 1-stage multivariate mixed-effect logistic regression model.A total of 437 studies were identified, of which 25 met our criteria for full-text analysis. Articles that measured VS with comparable methods were determined eligible for meta-analysis inclusion. The selected articles were highly heterogeneous in their use of grading scales and assessment of tumor size. Our review showed that size at diagnosis (odds ratio, 1.15; 95% confidence interval, 1.11-1.18; P0.0001) and intracanalicular localization (odds ratio, 0.49; 95% confidence interval, 0.26-0.90; P = 0.023) were associated with VS growth.The factors most frequently reported as being associated with growth within the literature were size of VS at diagnosis and localization of an intracanalicular component. Greater attention should be placed on these criteria within the surveillance imaging algorithm for VS.

Background Cowden syndrome (CS) is an autosomal dominant genodermatosis with a predisposition for the development of multiple cancers, benign hamartomas, and extracranial vascular malformations. Rarely, intracranial lesions like meningiomas and vascular malformations can also be present with CS. These vascular malformations include developmental venous anomalies, arteriovenous fistulae and cavernomas. Most cases of cavernomas are thought to be congenital, although in recent literature they have been shown to occur de novo with other conditions (e.g., other vascular malformations, trauma, postcranial surgery, viral infection, and genetic disorders). Case Description We present a 29-year-old woman who was diagnosed with Lhermitte-Duclos disease after episodes of persistent generalized headaches. She underwent a foramen magnum decompression and was subsequently diagnosed with CS. Ten years, later she was also diagnosed with 2 cerebral cavernomas that were not present on her prior monitoring scans. Conclusions We present a case of a patient with CS and LDD who had de novo cavernoma development several years after the initial diagnosis, as well as a review of the literature. We highlight the need of surveillance neuroimaging for patients with CS, as there is the risk of new development of vascular abnormalities (particularly cavernomas).

With the widespread use of imaging techniques, the possibility that an asymptomatic unruptured intracranial aneurysm (UIA) is detected has increased significantly. There is no established consensus regarding follow-up, duration, and frequency of such imaging surveillance. The objectives of this study include assessing the growth rate and rupture risk of small (less than 7mm) UIAs, identifying associated risk factors and providing an aneurysm surveillance protocol in appropriately selected patients. Systematic searches of Medline, Embase, and Cochrane Central were undertaken from database inception to March 2020 for published studies reporting the growth and rupture risks of small UIAs. Twenty-one studies reporting 8428 small UIAs were included in our meta-analysis. The pooled mean age was 61 years (95% CI: 55–67). The mean follow-up period for growth and rupture ranged from 11 to 108 months, with the pooled mean follow-up period across 14 studies being 42 months (95% CI: 33–51). Pooled overall growth rate was 6.0% (95% CI: 3.8–8.7). Pooled growth rates for aneurysms < 5mm and < 3 mm were 5.2% (95% CI: 3.0–7.9) and 0.8% (95% CI: 0.0–6.1), respectively. Pooled overall rupture rate was 0.4% (95% CI: 0.2–0.7). From the meta-regression analysis, having multiple aneurysms, smoking, hypertension, and personal history of SAH did not significantly predict growth, and a personal history of SAH, smoking, hypertension, and multiple aneurysms were not statistically significant predictors of rupture. Our findings suggest that small UIAs have low growth and rupture rates and very small UIAs have little or no risk for rupture. In the setting of incidental small UIAs, patients with multiple and/or posterior circulation aneurysms require more regular radiological monitoring.

Cavernous sinus haemangiomas (CSHs) are rare malformations of the microcirculation arising from the cavernous sinus. A systematic review and pooled data analysis of the associated clinical features, diagnostic modalities, management, and outcomes for CSHs was done. In total, 68 articles (338 cases) were eligible for analysis based on our selection criteria. The primary outcome measures were the occurrence of (i) and (ii) symptom resolution/improvement. Categorical outcome variables were assessed by binary logistic regression at 5% significance level. With headaches (39.9%) and diplopia (36.5%) as the most common presenting symptoms reported, dynamic contrast-enhanced MRI was the most commonly used diagnostic modality and was the most definitive pre-treatment imaging modality for diagnosing CSH with a sensitivity of 89.5%. The majority of CSHs were managed with radiosurgery (47.9% of cases), 37.9% by surgical resection alone, and 14.2% by a combination of both. Compared to patients that were treated with surgical resection only, those treated solely with radiosurgery had a 100% decrease in the odds of developing post-treatment complications (adjusted OR: 0.00, 95% CI: 0.00-0.002, p 0.001), with a 5.03 times greater odds of symptom resolution/improvement (adjusted OR: 5.03, 95% CI: 1.89-13.4, p = 0.001). Patients that underwent combined therapy had a 79% reduction in risk of developing post-treatment complications (adjusted OR: 0.21, 95% CI: 0.06-0.68, p = 0.01), with no statistically significant difference in the odds of symptom resolution/improvement, compared to those that had surgery only. In conclusion, radiosurgery offered the best outcomes with regards to symptom resolution/improvement and post-treatment complications in patients with CSH.

Background Traumatic Brain Injury (TBI) remains a significant problem in certain regions of the world but receives little attention despite its enormous burden. This discrepancy could consequently lead to various misconceptions among the general public. This study evaluated misconceptions about TBI in five African countries. Methods Data for this cross-sectional study were collected using the Common Misconception about Traumatic Brain Injury (CM-TBI) questionnaire, which was electronically disseminated from January 16 to February 6, 2021. Associations between the percentage of correct answers and independent variables (i.e., sociodemographic characteristics and experience with TBI) were evaluated with the ANOVA test. Additionally, answers to the question items were compared against independent variables using the Chi-Square test. A P-value

Background: Endoscopic skull base surgery (ESBS) is a new subspecialty area that has become more popular over the past 20 years. It is fast evolving and the indications are getting increasi...

Background During the coronavirus disease 2019 (COVID-19) pandemic, concerns have been raised regarding the increased risk of perioperative mortality for patients with COVID-19, and the transmission risk to healthcare workers, especially during endonasal neurosurgical operations. The Pituitary Society has produced recommendations to guide management during this era. We sought to assess contemporary neurosurgical practice and the effects of COVID-19. Methods A multicenter prospective observational cohort study was conducted at 12 tertiary neurosurgical units (United Kingdom and Ireland). Data were collected from March 23 to July 31, 2020, inclusive. The data points collected included patient demographics, preoperative COVID-19 test results, operative modifications, and 30-day COVID-19 infection rates. Results A total of 124 patients were included. Of the 124 patients, 116 (94%) had undergone COVID-19 testing preoperatively (transsphenoidal approach, 97 of 105 [92%]; expanded endoscopic endonasal approach, 19 of 19 [100%]). One patient (1 of 116 [0.9%]) had tested positive for COVID-19 preoperatively, requiring a delay in surgery until the infection had been confirmed as resolved. Other than transient diabetes insipidus, no other complications were reported for this patient. All operating room staff had worn at least level 2 personal protective equipment. Adaptations to surgical techniques included minimizing drilling, draping modifications, and the use of a nasal iodine wash. At 30 days postoperatively, no evidence of COVID-19 infection (symptoms or positive formal testing results) were found in our cohort and no mortality had occurred. Conclusions Preoperative screening protocols and operative modifications have facilitated endonasal neurosurgery during the COVID-19 pandemic, with the Pituitary Society guidelines followed for most of these operations. We found no evidence of COVID-19 infection in our cohort and no mortality, supporting the use of risk mitigation strategies to continue endonasal neurosurgery in subsequent pandemic waves.

Background CRANIAL (CSF Rhinorrhoea After Endonasal Intervention to the Skull Base) is a prospective multicenter observational study seeking to determine 1) the scope of skull base repair methods used and 2) corresponding rates of postoperative cerebrospinal fluid (CSF) rhinorrhea in the endonasal transsphenoidal approach (TSA) and the expanded endonasal approach (EEA) for skull base tumors. We sought to pilot the project, assessing the feasibility and acceptability by gathering preliminary data. Methods A prospective observational cohort study was piloted at 12 tertiary neurosurgical units in the United Kingdom. Feedback regarding project positives and challenges were qualitatively analyzed. Results A total of 187 cases were included: 159 TSA (85%) and 28 EEA (15%). The most common diseases included pituitary adenomas (n = 142/187), craniopharyngiomas (n = 13/187). and skull base meningiomas (n = 4/187). The most common skull base repair techniques used were tissue glues (n = 132/187, most commonly Tisseel), grafts (n = 94/187, most commonly fat autograft or Spongostan) and vascularized flaps (n = 51/187, most commonly nasoseptal). These repairs were most frequently supported by nasal packs (n = 125/187) and lumbar drains (n = 20/187). Biochemically confirmed CSF rhinorrhea occurred in 6/159 patients undergoing TSA (3.8%) and 2/28 patients undergoing EEA (7.1%). Four patients undergoing TSA (2.5%) and 2 patients undergoing EEA (7.1%) required operative management for CSF rhinorrhea (CSF diversion or direct repair). Qualitative feedback was largely positive (themes included user-friendly and efficient data collection and strong support from senior team members), demonstrating acceptability. Conclusions Our pilot experience highlights the acceptability and feasibility of CRANIAL. There is a precedent for multicenter dissemination of this project, to establish a benchmark of contemporary practice in skull base neurosurgery, particularly with respect to patients undergoing EEA.

Intracranial dural arteriovenous fistulas are defined as pathological anastomoses between meningeal arteries and dural venous sinuses or cortical veins. Rarely, they could drain into the venous system in and around the craniocervical junction and cause myelopathy and/or bulbar features. Due to the clinical and radiological features, prompt diagnosis poses a challenge, as there are several neurological differential diagnoses. Several mechanisms for the presentation have been considered, such as venous hypertension, direct compression by enlarged veins, and ischaemia due to infarcts or due to arterial steal. Digital subtraction angiography (DSA) is the gold-standard diagnosis for dural arteriovenous fistulas, where the visualisation of feeding arteries and the characterisation of venous drainage allows diagnosis and grading. Treatment options include conservative management, invasive (microsurgery) or minimally invasive options (transarterial or transvenous embolisation). We present a 32-year-old male who presented with myelopathy and bulbar features. Angiography demonstrated an intracranial dural arteriovenous fistula, which was successfully treated surgically.

Background: The endonasal transsphenoidal approach (TSA) has emerged as the preferred approach in order to treat pituitary adenoma and related sellar pathologies. The recently adopted expanded endonasal approach (EEA) has improved access to the ventral skull base whilst retaining the principles of minimally invasive surgery. Despite the advantages these approaches offer, cerebrospinal fluid (CSF) rhinorrhoea remains a common complication. There is currently a lack of comparative evidence to guide the best choice of skull base reconstruction, resulting in considerable heterogeneity of current practice. This study aims to determine: (1) the scope of the methods of skull base repair; and (2) the corresponding rates of postoperative CSF rhinorrhoea in contemporary neurosurgical practice in the UK and Ireland. Methods: We will adopt a multicentre, prospective, observational cohort design. All neurosurgical units in the UK and Ireland performing the relevant surgeries (TSA and EEA) will be eligible to participate. Eligible cases will be prospectively recruited over 6 months with 6 months of postoperative follow-up. Data points collected will include: demographics, tumour characteristics, operative data), and postoperative outcomes. Primary outcomes include skull base repair technique and CSF rhinorrhoea (biochemically confirmed and/or requiring intervention) rates. Pooled data will be analysed using descriptive statistics. All skull base repair methods used and CSF leak rates for TSA and EEA will be compared against rates listed in the literature. Ethics and dissemination: Formal institutional ethical board review was not required owing to the nature of the study–this was confirmed with the Health Research Authority, UK. Conclusions: The need for this multicentre, prospective, observational study is highlighted by the relative paucity of literature and the resultant lack of consensus on the topic. It is hoped that the results will give insight into contemporary practice in the UK and Ireland and will inform future studies.

Background Although several complications after ventriculoperitoneal shunting (VPS) have been reported, transoral protrusion of a peritoneal catheter is a rare event. In the few reported cases from the literature, it is more common in pediatric patients. This case report describes the first adult in the literature with a transoral VPS protrusion after jejunal perforation. Case Description A 58-year-old man with posttraumatic hydrocephalus was noted with the distal tubing of the VPS protruding from his mouth after he vomited. Radiologic imaging showed jejunal perforation of the distal part of the VPS. The VPS was initially externalized, and then removed. The patient remained stable. Conclusions We present a rare case of transoral protrusion of a peritoneal catheter 11 months after a VPS procedure and review of the literature through this article.

OBJECTIVEThe expanded endonasal endoscopic transsphenoidal approach has become increasingly used for craniopharyngioma surgery in the pediatric population, but questions still persist regarding its utility in younger children, in recurrent and irradiated tumors, and in masses primarily located in the suprasellar region. The narrow corridor, incomplete pneumatization, and fear of hypothalamic injury have traditionally relegated this approach to application in older children with mostly cystic craniopharyngiomas centered in the sella. The authors present a series of consecutive pediatric patients in whom the endonasal endoscopic approach was used to remove craniopharyngiomas from patients of varied ages, regardless of the location of the tumor and previous treatments or surgeries, to ascertain if the traditional concerns about limitations of this approach are worth reevaluatingMETHODSEleven consecutive pediatric patients (age ≤ 18 years) underwent surgery via an endoscopic transsphenoidal approach at NewYork-Presbyterian/Weill Cornell Medical Center from 2007 to 2016. The authors recorded the location, consistency, and size of the lesion, assessed for hypothalamic invasion radiographically, calculated skull base measurements, and assessed parameters such as extent of resection, visual function, endocrinological function, weight gain, and return-to-school status.RESULTSThe average age at the time of surgery was 7.9 years (range 4–17 years) and the tumor sizes ranged from 1.3 to 41.7 cm3. Five cases were purely suprasellar, 5 had solid components, 4 were reoperations, and 5 had a conchal sphenoid aeration. Nevertheless, gross-total resection was achieved in 45% of the patients and 50% of those in whom it was the goal of surgery, without any correlation with the location, tumor consistency, or the age of the patient. Near-total resection, subtotal resection, or biopsy was performed intentionally in the remaining patients to avoid hypothalamic injury. Anterior pituitary dysfunction occurred in 81.8% of the patients, and 63.3% developed diabetes insipidus . Two patients (18%) had a greater than 9% increase in body mass index. Visual function was stable or improved in 73%. All children returned to an academic environment, with 10 of them in the grade appropriate for their age. There was a single case of each of the following: CSF leak, loss of vision unilaterally, and abscess.CONCLUSIONSThe endoscopic transsphenoidal approach is suitable for removing pediatric craniopharyngiomas even in young children with suprasellar tumors, conchal sphenoid sinus, recurrent tumors, and tumors with solid components. The extent of resection is dictated by intrinsic hypothalamic tumor invasiveness rather than the approach. The endoscopic transsphenoidal approach affords the ability to directly inspect the hypothalamus to determine invasion, which may help spare the patient from hypothalamic injury. Irrespective of approach, the rates of postoperative endocrinopathy remain high and the learning curve for the approach to a relatively rare tumor is steep.

We present a 69-year-old transgender woman who underwent gender-affirming surgery in 1998 and gender-affirming hormone therapy (cyproterone acetate (CPA) and estradiol) since this time. Following an MRI scan to investigate tremor in 2013, an incidental left anterior clinoid and right petrous meningioma were identified. Subtotal surgical resection was achieved for the anterior clinoid meningioma (WHO grade 1, meningothelial subtype). At follow-up in 2016, an olfactory groove meningioma and left greater wing of sphenoid meningioma were identified. By 2017, both tumours, along with the petrous meningioma, demonstrated significant growth. In 2018, clinical decline was evident and MRI demonstrated further tumour growth. Surgery was scheduled and the olfactory groove meningioma was completely resected (WHO grade 2, chordoid subtype). Hormones were stopped, after which regression of the petrous meningioma was observed. This case demonstrates an association between high-dose CPA and estradiol and the development, growth and regression of meningiomas in a transgender woman.

Objective:To prospectively evaluate the incidence, risk factors and natural history of postoperative cerebral venous sinus thrombosis (pCVST) in translabyrinthine vestibular schwannoma microsurgical resection and propose a potential management paradigm.Study Design:Prospective, single cohort, multicenter study.Setting:State-wide academic tertiary referral centers.Patients:Fifty-four consecutive patients who underwent translabyrinthine vestibular schwannoma resection.Main Outcome Measures:Incidence of pCVST on postoperative imaging on Day 7, Day 28, and 12 months postoperatively. Patients and tumor characteristics, risk factors, length of stay, intraoperative parameters, complications, and follow-up were analyzed.Results:pCVST was demonstrated in 21 patients (38.9%) on postoperative imaging. All patients with pCVST were treated conservatively and remained asymptomatic in the immediate postoperative period and long-term follow-up. There were no instances of venous infarction, intracranial hemorrhage, or neurological deficits. A majority (61.1%) of pCVST recannalised on long-term follow up with conservative management. There was a statistical association with pCVST and surgery on the side of the non-dominant cerebral venous drainage (n=17, 80.1%, p=0.034). Patients with pCVST were significantly more likely to have a postoperative cerebrospinal fluid (CSF) leak (n=5, 23.8%, p=0.017).Conclusion:The incidence of pCVST following translabyrinthine vestibular schwannoma resection is much higher than previously recognized. pCVST is more likely to occur when surgery is performed on tumors situated on the side of non-dominant cerebral venous drainage. Despite the high prevalence of this iatrogenic phenomenon, all patients were asymptomatic and a majority resolved, thereby suggesting that conservative management is safe. Correlation between pCVST and increased incidence of CSF leak requires further investigation.

Background Recurrent artery of Heubner (RAH) aneurysms are rare, with only 7 reported cases in the literature to date. In evaluating cerebral aneurysms, cerebral digital subtraction angiogram (DSA) is considered the gold standard and demonstrated the RAH aneurysms in previous case reports. We present a case of spontaneous subarachnoid hemorrhage secondary to RAH aneurysmal rupture, with initial DSA misleading, suggesting minor aneurysmal filling of a presumed thrombosed A1 segment aneurysm instead. Case Description A 71-year-old female presented with sudden-onset severe headache, nausea, and vomiting. Computed tomography along with computed tomography angiogram revealed a left-sided subarachnoid hemorrhage and a 5-mm aneurysm arising from either the left A1 or distal internal carotid artery. In addition, complex anterior cerebral artery anatomy with trifurcation was noted. This along with partial aneurysmal thrombosis made identification of the anatomy difficult on subsequent DSA, though a residual neck was still suggested to be arising from A1. Intraoperatively, the aneurysm was found to be arising from the RAH instead and was successfully secured by clipping. Conclusions This case highlights the risk of RAH aneurysms masquerading as emanating from the A1 segment on DSA. Case particularities of complex anatomy and aneurysmal thrombosis were contributory. Other factors such as severe vasospasm, microaneurysms, and aneurysmal compression by overlying hematoma or brain parenchymal swelling could also distort DSA interpretation. These are important considerations in treatment planning by neurovascular surgeons and endovascular neuroradiologists.

We report the case of a large osteoblastoma arising in the frontal bone of a 20-year-old female. The lesion was first noted after a fall, and grew steadily in size following further head injury during pregnancy. Initial plain radiography demonstrated an area of radiolucency, with subsequent cross-sectional imaging revealing the extent of the lesion. Following successful surgical resection, histological features were suggestive of an aggressive osteoblastoma with aneurysmal bone cyst-like changes. We consider the influence of pregnancy and trauma on osteoblastoma behavior.

Background: Ventral brainstem compression secondary to complex craniovertebral junction abnormality is an infrequent cause of neurologic deterioration in pediatric patients. However, in cases of symptomatic, irreducible ventral compression, 360° decompression of the brainstem supported by posterior stabilization may provide the best opportunity for improvement in symptoms. More recently, the endoscopic endonasal corridor has been proposed as an alternative method of odontoidectomy associated with less morbidity. We report the largest single case series of pediatric patients using this dual-intervention surgical technique. The purpose of this study was to evaluate the surgical outcomes of pediatric patients who underwent posterior occipitocervical decompression and instrumentation followed by endoscopic endonasal odontoidectomy performed to relieve neurologic impingement involving the ventral brainstem and craniocervical junction. Methods: Between January 2011 and February 2017, 7 patients underwent posterior instrumented fusion followed by endonasal endoscopic odontoidectomy at our unit. Standardized clinical and radiological parameters were assessed before and after surgery. A univariate analysis was performed to assess clinical and radiologic improvement after surgery. Results: A total of 14 operations were performed on 7 pediatric patients. One patient had Ehlers-Danlos syndrome, 1 patient had a Chiari 1 malformation, and the remaining 5 patients had Chiari 1.5 malformations. Average extubation day was postoperative day 0.9. Average day of initiation of postoperative feeds was postoperative day 1.0. Conclusions: The combined endoscopic endonasal odontoidectomy and posterior decompression and fusion for complex craniovertebral compression is a safe and effective procedure that appears to be well tolerated in the pediatric population. Key words: Axis, Basilar invagination, Chiari, Endonasal, Endoscopic, Odontoidectomy, Pediatric

OBJECTIVEAnterior skull base meningiomas are benign lesions that cause neurological symptoms through mass effect on adjacent neurovascular structures. While traditional transcranial approaches have proven to be effective at removing these tumors, minimally invasive approaches that involve using an endoscope offer the possibility of reducing brain and nerve retraction, minimizing incision size, and speeding patient recovery; however, appropriate case selection and results in large series are lacking.METHODSThe authors developed an algorithm for selecting a supraorbital keyhole minicraniotomy (SKM) for olfactory groove meningiomas or an expanded endoscopic endonasal approach (EEA) for tuberculum sella (TS) or planum sphenoidale (PS) meningiomas based on the presence or absence of olfaction and the anatomical extent of the tumor. Where neither approach is appropriate, a standard transcranial approach is utilized. The authors describe rates of gross-total resection (GTR), olfactory outcomes, and visual outcomes, as well as complications, for 7 subgroups of patients. Exceptions to the algorithm are also discussed.RESULTSThe series of 57 patients harbored 57 anterior skull base meningiomas; the mean tumor volume was 14.7 ± 15.4 cm3 (range 2.2–66.1 cm3), and the mean follow-up duration was 42.2 ± 37.1 months (range 2–144 months). Of 19 patients with olfactory groove meningiomas, 10 had preserved olfaction and underwent SKM, and preservation of olfaction in was seen in 60%. Of 9 patients who presented without olfaction, 8 had cribriform plate invasion and underwent combined SKM and EEA (n = 3), bifrontal craniotomy (n = 3), or EEA (n = 2), and one patient without both olfaction and cribriform plate invasion underwent SKM. GTR was achieved in 94.7%. Of 38 TS/PS meningiomas, 36 of the lesions were treated according to the algorithm. Of these 36 meningiomas, 30 were treated by EEA and 6 by craniotomy. GTR was achieved in 97.2%, with no visual deterioration and one CSF leak that resolved by placement of a lumbar drain. Two patients with tumors that, based on the algorithm, were not amenable to an EEA underwent EEA nonetheless: one had GTR and the other had a residual tumor that was followed and removed via craniotomy 9 years later.CONCLUSIONSUtilizing a simple algorithm aimed at preserving olfaction and vision and based on maximizing use of minimally invasive approaches and selective use of transcranial approaches, the authors found that excellent outcomes can be achieved for anterior skull base meningiomas.

The fossa navicularis is an anatomical variant of the skull base thought to be a rare finding. It represents a bony depression in the skull base. The authors here report the case of a fossa navicularis magna in a 9-year-old female who had been treated for recurrent episodes of meningitis.A literature review was also done to highlight the unique features and clinical importance of this distinctive radiological skull base finding. The literature search covered papers from the 19th century up to 2018. Earlier authors described “fossa navicularis” as a very rare skull base finding. So far, only three cases of fossa navicularis with associated clival or intracranial infection have been reported in the literature. This is the fourth reported case, and the defect was closed endoscopically via a transnasal route. This morphological skull base anomaly should be considered in the differential diagnoses for an unexplained skull base infective pathology.Skull base surgeons should be aware of the existence of the fossa navicularis because of its clinical importance in rendering a prompt diagnosis and appropriate treatment.

Background Basilar invagination can result from systemic diseases that can weaken structural integrity of the craniocervical junction. Definitive treatment often requires ventral decompression and posterior decompression and fusion. Endonasal odontoidectomy is a relatively new minimal access procedure; quality of life (QOL) after this procedure has not been reported. Methods We reviewed a consecutive database of endonasal odontoidectomy cases and identified patients having posterior decompression and fusion. Two QOL questionnaires were administered postoperatively: Sino-Nasal Outcome Test and 36-Item Short Form Survey. Comparisons with other endonasal or Chiari procedures were performed. Results The study comprised 14 patients; 79% had Chiari malformation in addition to basilar invagination. Mean follow-up was 17.2 months. Symptomatic improvement occurred in 78.6% after surgery. Average postoperative Sino-Nasal Outcome Test scores were 39.2 ± 17.93, with worst scores in areas related to fatigue and sleep patterns but not nasal function. 36-Item Short Form Survey scores were lower in areas of physical function and general health but better for emotional health and pain. Compared with patients undergoing Chiari malformation surgery without endonasal odontoid resection or fusion, patients undergoing odontoidectomy had higher QOL in areas of role emotional, emotional well-being, and pain but worse QOL in general health and role physical. Conclusions Patients undergoing posterior decompression and fusion with endonasal odontoidectomy do well after surgery with respect to nasal function and emotional health. Patients who also have severe basilar invagination associated with systemic diseases demonstrate reduced QOL after surgery in areas of physical function and sleep leading to fatigue, irritability, and concentration difficulty, likely related to their systemic disease.

Meningiomas are the most common primary brain tumor, and the knowledge of these tumors and their management has understandably evolved over the years. With better and more affordable radiologic investigations and changes to the WHO classification grading, there has been a stark increase in diagnosed cases. As common as meningiomas are, there is still a paucity of related clinical trials to serve as a basis for evidence-based algorithms to guide management. The treatment of choice for meningiomas is total resection, but in about 50% of cases this is not achievable due to their locations (commonly, the meningiomas associated with the skull base) or the patient's comorbidities. With improving technologies, it is hoped that there will be easier and better ways of dealing with this surgical dilemma.

Craniopharyniomas arise from the sellar region and are particularly challenging because of their close proximity to critical neurovascular structures, including cranial nerves, brainstem, internal carotid arteries, posterior cerebral arteries, hypothalamus, and the pituitary gland. The tumors are benign on histology but can cause serious symptoms by compression of surrounding vital structures. While radical surgery and gross total resection (GTR) remains the first line treatment, choosing the optimal surgical approach is critical for the minimization of complications and postoperative morbidity. The extended endoscopic endonasal approach (EEEA) offers a direct route towards the sellar and suprasellar regions and has proven to be a safe and effective for GTR, with lower rates of complications and postoperative morbidity than open transcranial approaches. However, traditional transcranial approaches remain relevant in tumors with significant lateral extension. Radiotherapy can be considered in cases of subtotal resection (STR) or if surgery is not possible, although progressive growth of cysts with neurological deterioration has been demonstrated no infrequently. Newly developed chemotherapy for papillary craniopharygniomas might expand treatment options in the future.

Double adenomas in the pituitary gland are a rare occurrence. The ability to cure a hormone-producing adenoma with surgery is dependent on the ability to identify and completely remove the correct adenoma. The relative frequency of each subtype of hormone-secreting adenomas confirmed with magnetic resonance image (MRI), surgery and immunohistochemistry is not defined. Following PRISMA guidelines, we performed a systematic review of PubMed Central, Google Scholar, Scopus Database, Cochrane database and Science Research, using the key-words: “double pituitary adenomas”, “multiple pituitary adenomas” and only papers where multiple or truly separate double pituitary adenomas were identified pre-surgically by MRI and/or confirmed by pathology/immunohistochemistry staining were included. We reviewed papers from 1975 to 2016 and found 17 cases with two pituitary adenomas and 1 with three pituitary adenomas. The ages ranged from 22 to 67 years old, and there were twelve females and five males. Immunohistochemical staining showed that the most common double pituitary adenomas were growth hormone (GH)- followed by adrenocorticotropic (ACTH)-secreting. Double pituitary adenomas are rare but most commonly found with GH- or ACTH-producing tumors. It is critical to remove all identified possible adenomas to achieve biochemical cure and clinicians should have heightened sensitivity in patients with acromegaly or Cushing’s Disease.

We present the case of a brain abscess caused by a combination of rare organisms (Trueperella bernardiae and Peptoniphilus harei) in a patient with chronic suppurative otitis media that had been complicated by the presence of a cholesteatoma. The authors believe this is the first report published in the literature.